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Consensus Guidelines for Managing Gynecological and Obstetric Care in Women With Hereditary Angiooedema Resulting From C1-Inhibtor Deficiency: Presented at AAAAI

By Bryan DeBusk

SAN DIEGO, CA -- February 26, 2007 -- Clinicians caring for women with hereditary angiooedema (HAE) should give special attention to addressing this group's gynecological and obstetrical needs, according to new guidelines unveiled here at the 2007 American Academy of Allergy, Asthma, and Immunology (AAAAI) annual meeting.

The European C1-INH Deficiency Working Group (PREHAEAT) developed guidelines based on consultations with gynecologists, a comprehensive survey of the literature, and a survey of hormonal problems in 150 female HAE patients.

"There are some doubts that all clinicians have when dealing with these patients," said the PREHAEAT coordinator for consensus, Teresa Caballero, MD, PhD, department of allergy, Hospital Universitario La Paz, Madrid, Spain. Citing difficulty finding literature that describes appropriate treatment for gynecological and obstetric needs in women with HAE, Dr. Caballero and her colleagues developed a consensus document of clinical guidelines.

The consensus guidelines include restrictions on the use of some therapies during pregnancy and breastfeeding, limiting the range of appropriate contraceptives, and identifying diagnostic and surgical procedures requiring prophylactic C1-inhibitor transfusions.

The group completed work in November 2006 and is preparing its report for publication.

"The clinician can go to this paper when it's published and can see what to do," Dr. Caballero said in a presentation on February 24^th. The PREHAEAT group plans to make the complete report available online following publication.

Highlights from the guidelines:

· Virilisation, menstrual irregularities, hirsutism, and other adverse effects of attenuated androgen (AA) therapy may be countered with 100 to 200 ng of spironolactone per day, but this may reduce the efficacy of the AA therapy.
· 80% of patients report worsening of symptoms with oestrogens and their use should be avoided.
· Contraception should be limited to progesterone-only formulations or barrier devices.
· Angiotensin II antagonists (A-A) therapy should be discontinued during pregnancy or lactation, and tranexamic acid or human C1-inhibitor concentrate may be used as a replacement.
· Prophylactic transfusion of human C1-inhibitor concentrate should be used prior to amniocentesis or salpingography, prior to the use of oestrogens to stimulate egg release for in vitro fertilisation, or prior to delivery by Caesarean section.
· Vaginal deliveries do not generally require prophylaxis, but C1 inhibitor should be available in the delivery suite as 6% of HAE patients report attacks during or just after delivery.
· Patients with breast cancer should discontinue A-A therapy.
· Surgical procedures should be conducted under local anaesthesia where possible and prophylactic C1 inhibitor should be used.


[Poster title: European Consensus for Gynecological and Obstetric Management of Women With Hereditary Angioedema due to C1-Inhibitor Deficiency (HAE): PREHAEAT. Abstract 43]

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